Date: Sunday, November 8, 2015
Session Type: ACR Poster Session A
Session Time: 9:00AM-11:00AM
Differentiating between primary Sjӧgren’s syndrome (PSS) with systemic manifestations (SM), and Sjӧgren’s -SLE overlap syndrome (OL) can be a clinical challenge. Approximately 30 % of PSS patients have systemic manifestations, while 30 % of SLE patients display sicca symptoms and are SS-A/B positive. This study was undertaken to determine if the prevalence and pattern of SM can differentiate PSS with SM from OL.
Charts of 118 patients were retrospectively evaluated to compare the prevalence of SM, and association of diagnosis with twelve categories of SM. Of 118 patients, 91 were classified as PSS and 27 were classified as OL. All PSS patients met AECG criteria for PSS, and had two or fewer ACR SLE criteria. OL patients were defined as having greater than two AECG criteria, and greater than two SLE criteria. The cohorts were also compared with respect to serological features. Two-sample test and Wilcoxon rank sum test were used to test for association between SM and diagnostic category. All associations with p<0.3 were further tested using an adjusted logistic regression model.
There were no significant differences in demographics between PSS and OL patients. However, in the adjusted model white race was more common 4.09 times more common in PSS. There were no significant differences in frequency of xerostomia and xeropthalmia between PSS and OL. Neurologic disease was the most prevalent SM in PSS (43 %), whereas arthritis was most common in OL (52%). There were significant differences in association between PSS and OL with regards to arthritis (p<0.001), leukopenia (p=0.009), thrombocytopenia (p=0.001), and serositis (p=0.037) . There was no significant difference in prevalence of renal manifestations. There also were similar rates of Raynaud’s. Positivity for SS-A, and SS-B was similar. Overall, patients with OL had a higher median number of EGM than patients with PSS (p=0.009).
PSS and OL patients are similar with regards to age of presentation and gender, but the two groups differ significantly in ethnicity with non-Caucasian race more likely to develop overlap features with SLE. Xerostomia and xeropthalmia cannot differentiate between these groups. Inflammatory arthritis, leukopenia, thrombocytopenia, and serositis may assist in differentiating between PSS and OL. Lower than expected rates of renal disease in the OL group suggest a possible modifying effect of the Sjӧgren’s phenotype. SS-A, and SS-B as well as Raynaud’s do not appear to discriminate between these two disease states.
To cite this abstract in AMA style:Carsons SE, Movva S, Ienopoli S, Aziz S, Gul M, Calixte R, Brodsky T. Can Extraglandular Manifestations Differentiate Primary Sjögren’s Syndrome from Sjögren’s-Systemic Lupus Erythematosus Overlap Syndrome? [abstract]. Arthritis Rheumatol. 2015; 67 (suppl 10). https://acrabstracts.org/abstract/can-extraglandular-manifestations-differentiate-primary-sjogrens-syndrome-from-sjogrens-systemic-lupus-erythematosus-overlap-syndrome/. Accessed December 5, 2021.
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ACR Meeting Abstracts - https://acrabstracts.org/abstract/can-extraglandular-manifestations-differentiate-primary-sjogrens-syndrome-from-sjogrens-systemic-lupus-erythematosus-overlap-syndrome/